The difference of disease perception by juvenile idiopathic arthritis patients and their parents : analysis of the JAMAR questionnaire.

BACKGROUND: The JAMAR (Juvenile Arthritis Multidimensional Assessment Report) has been developed to evaluate the perception of the patient and his parents on different items: well-being, pain, functional status, quality of life, disease activity, disease course, side effects of medication, therapeutic compliance and satisfaction with illness outcome. Our aim was to compare disease's perception by JIA patients and their parents. METHODS: We included into the study 100 consecutive patients over 7 years of age. We asked both parent and child to complete the JAMAR questionnaire. For each patient we recorded demographic and disease related data. We examined the level of disagreement between children and parents for the quantitative items of the JAMAR: VAS Pain, VAS Disease Activity, VAS Well Being, Juvenile Arthritis Functional Score, HRQoL. Then we looked for a relation between discordance-rate and demographic and clinical variables. RESULTS: Children and parents' median scores for all five items were similar. Individual dyads agreement was low, with a large amount of pairs (80) discordant for at least one item. We found higher MD VAS and JADAS in more discordant dyads, suggesting that when the disease is more active discordance between child and parent increase. CONCLUSION: The JAMAR questionnaire is an important tool that helps clinicians to detect divergent child and parent's disease perceptions. It is essential that both patients and parents fill the JAMAR questionnaire for a complete clinical and psychosocial evaluation

Neonatal treatment of CINCA syndrome.

ABSTRACT: Chronic Infantile Neurological Cutaneous Articular (CINCA) syndrome, also called Neonatal Onset Multisystem Inflammatory Disease (NOMID) is a chronic disease with early onset affecting mainly the central nervous system, bones and joints and may lead to permanent damage. We report two preterm infants with severe CINCA syndrome treated by anti-interleukin-1 in the neonatal period, although, so far, no experience with this treatment in infants younger than three months of age has been reported. A review of the literature was performed with focus on treatment and neonatal features of CINCA syndrome. CASE REPORT: Two cases suspected to have CINCA syndrome were put on treatment with anakinra in the early neonatal period due to severe clinical presentation. We observed a rapid and persistent decline of clinical signs and systemic inflammation and good drug tolerance. Diagnosis was confirmed in both cases by mutations in the NLRP3/CIAS1-gene coding for cryopyrin. As particular neonatal clinical signs polyhydramnios and endocardial overgrowth are to be mentioned. CONCLUSION: We strongly suggest that specific treatment targeting interleukin-1 activity should be started early. Being well tolerated, it can be introduced already in neonates presenting clinical signs of severe CINCA syndrome in order to rapidly control inflammation and to prevent life-long disability

Chiral Surface States in the Bulk Quantum Hall Effect

In layered samples which exhibit a bulk quantum Hall effect (QHE), a two-dimensional (2d) surface ``sheath" of gapless excitations is expected. These excitations comprise a novel 2d chiral quantum liquid which should dominate the low temperature transport along the field (z-axis). For the integer QHE, we show that localization effects are completely absent in the ``sheath", giving a metallic z-axis conductivity. For fractional filling $\nu =1/3$, the ``sheath" is a 2d non-Fermi liquid, with incoherent z-axis transport and $\sigma_{zz} \sim T^3$. Experimental implications for the Bechgaard salts are discussed.Comment: 4 pages, RevTeX 3.0, with two encapsulated postscript figures, which can be automatically included in-text if desired. The complete postscript file is available on the WWW at http://www.itp.ucsb.edu/~balents/sheath.p

Edge electron states for quasi-one-dimensional organic conductors in the magnetic-field-induced spin-density-wave phases

We develop a microscopic picture of the electron states localized at the edges perpendicular to the chains in the Bechgaard salts in the quantum Hall regime. In a magnetic-field-induced spin-density-wave state (FISDW) characterized by an integer N, there exist N branches of chiral gapless edge excitations. Localization length is much longer and velocity much lower for these states than for the edge states parallel to the chains. We calculate the contribution of these states to the specific heat and propose a time-of-flight experiment to probe the propagating edge modes directly.Comment: 4 pages, 2 figures. V.2: Minor changes to the final version published in PR

Endogenous mediators of growth

In the present review it is not possible to discuss the effects of the numerous endogenous mediators of growth. What we have attempted to do is to indicate the areas of controversy and the need for further research. In our view, four main questions arise. First, what are the relative contributions of the direct and indirect effects of GH? Indeed, if GH can produce all its effects by local production of IGF, is the original somatomedin hypothesis still tenable? Second, how is the biological activity of the IGF modified by the presence of binding proteins? Because of the role of binding proteins in modulating IGF bioactivity, care must be taken when interpreting results from immunoassays for IGF because this will only represent the concentration of IGF not the level of biological activity, a situation which is analogous to that which pertains with certain polypeptide hormones (for review, see Robertson et al. 1987). Third, how are the activities of the osteoblast and osteoclast coupled so that in the mature adult, bone formation and bone resorption are roughly equivalent? Understanding of this process will undoubtedly involve the elucidation of the roles and interactions between a number of locally acting growth factors and systemic hormones and will lead to the understanding of certain metabolic bone diseases such as osteoporosis. Last, how is the response to local stimuli such as mechanical stress transduced? This is again probably dependent on the activity of local growth factors but may also involve changes in the interactions between bone cells and their underlying matrix components (Skerry et al. 1988)

Hot Spots and Transition from d-Wave to Another Pairing Symmetry in the Electron-Doped Cuprate Superconductors

We present a simple theoretical explanation for a transition from d-wave to another superconducting pairing observed in the electron-doped cuprates. The d_{x^2-y^2} pairing potential Delta, which has the maximal magnitude and opposite signs at the hot spots on the Fermi surface, becomes suppressed with the increase of electron doping, because the hot spots approach the Brillouin zone diagonals, where Delta vanishes. Then, the d_{x^2-y^2} pairing is replaced by either singlet s-wave or triplet p-wave pairing. We argue in favor of the latter and discuss experiments to uncover it.Comment: 6 pages, 4 figures, RevTeX 4. V.2: Extra figure and many references added. V.3: Minor update of references for the proof

Integer Quantum Hall Effect in Double-Layer Systems

We consider the localization of independent electron orbitals in double-layer two-dimensional electron systems in the strong magnetic field limit. Our study is based on numerical Thouless number calculations for realistic microscopic models and on transfer matrix calculations for phenomenological network models. The microscopic calculations indicate a crossover regime for weak interlayer tunneling in which the correlation length exponent appears to increase. Comparison of network model calculations with microscopic calculations casts doubt on their generic applicability.Comment: 14 pages, 12 figures included, RevTeX 3.0 and epsf. Additional reference

Ogden Material Calibration via Magnetic Resonance Cartography, Parameter Sensitivity, and Variational System Identification

Contemporary techniques in the mechanical calibration of materials that leverage full three-dimensional deformation fields and the weak form of the equilibrium equations face challenges in the numerical solving procedure of the inverse characterization problem. As material models and descriptions differ, so too must the approaches for identifying their system mechanics. The widely-used Ogden material model, comprised of an unknown number of terms of the same mathematical form, presents challenges in interpretability, stability, and parsimony. In turn, we intend to use our estimates to assess and improve our experimental design. Using fully 3D displacement fields acquired in silicone elastomers using our recently-developed magnetic resonance cartography (MR-u) technique on the order of $>20,000$ points per sample, we leverage PDE-constrained optimization as the basis of variational system identification of our material parameters. We incorporate the statistical F-test to maintain parsimony of representation. Using a new decomposition of the deformation field locally into mixtures of biaxial and uniaxial tensile states, we evaluate experiments based on an analytical sensitivity metric, and discuss the implications for future experimental design.Comment: 16 pages, 7 figure

A 6-month, multicenter, open-label study of fixed dose naproxen/esomeprazole in adolescent patients with juvenile idiopathic arthritis

© 2018 The Author(s). Background: Juvenile idiopathic arthritis (JIA) is an inflammatory arthritis of unknown etiology, which lasts for greater than 6 weeks with onset before 16 years of age. JIA is the most common chronic rheumatic disease in children. NSAIDs have been the mainstay of initial management with naproxen (NAP) being commonly used, but they may cause serious side effects such as gastric ulcers which can be reduced by concomitant administration of proton pump inhibitors, such as esomeprazole (ESO). Methods: Primary objective was to evaluate the safety and tolerability of 3 fixed doses of NAP/ESO in JIA patients aged 12 to 16 years. Forty-six children and adolescents with JIA by International League of Associations for Rheumatology criteria, mean age of 13.6 years, from 18 US sites were prospectively enrolled over 2 years and followed for up to 6 months. Doses of the NAP/ESO fixed combination were based on baseline weight. The exploratory efficacy outcome was assessed with the ACR Pediatric-30, - 50, - 70, - 90 Response and the Childhood Health Assessment Questionnaire (CHAQ) discomfort and functional scores at months 1, 3, and 6 as change from baseline. Occurrence and causality were assessed for treatment emergent AEs (TEAEs) and discontinuations were monitored monthly. Results: Forty-six patients received at least 1 dose of naproxen/esomeprazole and 36 completed the trial. Thirty-seven (80.4%) had at least 1 treatment emergent adverse event (TEAE) and, with the exception of 2 events in one patient, all of the TEAEs were mild or moderate. Frequent TEAEs (≥5% of patients) were upper respiratory tract and gastrointestinal related. Eleven (23.9%) had at least 1 TEAE considered to be related to study drug. Four patients (8.7%) discontinued due to a TEAE with one of these being the only serious AE reported, acute hepatitis. Mean number of active joints at baseline was 3.1. Improvement in JIA signs and symptoms occurred at most assessments and by month 6, the percentage of patients with an ACR Pediatric-30, - 50, - 70, and - 90 Response was 47.1, 38.2, 32.4, and 17.6%, respectively. The percent of patients achieving ACR Pediatric response increased over time. CHAQ discomfort improved at each assessment and functional scores improved at all assessments for \u27Arising, Walking, and Activities\u27 with several improved for \u27Dressing and Grooming, Eating, Hygiene, and Grip\u27. There was no indication of a dose-related efficacy effect. Conclusion: NAP/ESO was well tolerated in JIA patients aged 12 to 16 years with high levels of response to ACR criteria. No new safety signals were identified for the well-characterized components of this fixed dosed JIA treatment, which was developed to reduce the risk of gastric ulcers